PB2057: REAL -WORLD OVERALL SURVIVAL OF MYELOFIBROSIS PATIENTS ON RUXOLITINIB – A 6 YEAR ANALYSIS IN ALBANIAN POPULATION
Authors: A. Perolla, P. Pulluqi, A. Cili, E. Calliku, T. Caja, A. Ivanaj
Journal: HemaSphere (2022)

Background: The survival and the outcome of the patients with Myelofibrosis has been prolonged with the use of Janus kinase inhibitors. The long term follow -up of the patients with primary /secondary Myelofibrosis treated with ruxolitinib demonstrated through several clinical trials an improvement in overall survival and in the quality of life of those patients. The use of IPSS risk classification in Myelofibrosis has played an important role in the prognostic stratification of the patients predictioning also the survival. Aims: Our study aims to evaluate the Overall Survival (OS) of all the patients diagnosed with Myelofibrosis and treated with Ruxolitinib from 2015 until january 2022. We also aimed to evaluate the role of IPSS risk group classification in the outcome and survival of MF patients in the Albanian population. Methods: We selected retrospectively all the patients diagnosed in our University Hospital Center “Mother Teresa” in Tirana, Albania with primary and secondary Mielofibrosis who started the treatment with ruxolitinib from 2015 until January 2022. We collected the data from the registries and database of Hematology Clinic based on the first availability of ruxolitinib in Albania since 2015. The data were analysed with SPSS Statistics 25.01. Results: From 2015 until January 2022 we have treated with Ruxolitinib 103 patients diagnosed with Myelofibrosis, 87 (84.5%) were primary MF and 16 (15.5%) were secondary MF. The median age resulted of 64 (range 31 – 88) years old. According to the IPSS risk group classification 1(!%) resulted IPSS Low risk, 21 (20.4%) were IPSS Int-1 risk, 49 (47.6%) resulted IPSS Int-2 risk, and 32(31.1%) were IPSS High risk. Based on the presence of 0 (low risk), 1 (intermediate risk-1), 2 (intermediate risk-2) or greater than or equal to 3 (high risk) of IPSS, we delineated 4 risk groups with no overlapping in their survival curves and the respective median survivals were 75, 40, 33, and 24 months We classified as IPSS Low risk all the patient with IPSS low and Int-1 risk, and as High risk all the patients with IPSS Int-2 and High risk. We identified 22 (21.4%) low risk patients and 81(78.7%) high risk patients. We compared the median overall survival between the Low risk and High risk MF groups and concluded that median OS in Low risk MF resulted 47 months (95% Confidence interval (CI): 11.2-82.7) and in High risk MF resulted of 32 months (95% Confidence interval (CI): 3,9 – 39,7) with P<0.01. Image:Summary/Conclusion: Myelofibrosis (MF) is a chronic myeloproliferative neoplasm who can develop de novo (primary MF) or secondary in patients with antecedent polycythemia vera (PV) or essential thrombocythemia (ET). The mean age at the moment of diagnosis is 65 year old. In our study me median age at the moment of the diagnosis resulted 64 years old. In our study we compared the median overall survival between the Low risk and High risk MF groups and concluded that median OS in Low risk MF resulted 47 months (95% Confidence interval (CI): 11.2-82.7) and in High risk MF resulted of 32 months (95% Confidence interval (CI): 3,9 – 39,7) with P<0.01Despite the established fact on efficacy of Ruxolitinib in MF patients, real -world data still are limited. Our objective was to analyse the overall survival of our patients with Mielofibrosis under treatment with ruxolitinib, however additional data are needed.